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Medical Report:

Digestive System (Gut, Gastro-intestinal) Involvement in Scleroderma

Updated January 2019

By Dinesh Khanna, MD, MS Professor of Medicine Director, University of Michigan Scleroderma Program

http://bit.ly/UMSclerodermaResearch https://www.selfmanagescleroderma.com

1 fter the skin, the digestive system is the most com­ Table 1: Frequency of GI symptoms A monly affected system (from 209 patients) in people with scleroderma, affect­ ing about 75 to 90 percent of all Prevalence patients (Table 1 and Figure 1). Symptom (%) The majority of people with gut • Heartburn 71 involvement experience symptoms • Trouble 52 that interfere with their day-to-day • Bloating 80 activities and quality of life. • Diarrhea 51 The function of the gut is to push • Constipation 51 the food and liquid down from the • Fecal Incontinence 35 to the (or colon), extract and absorb nutrients, Scleroderma Foundation survey 2010 and excrete the waste in the form of stool. It does so by well-orchestrated mouth moist and use over-the- The is the area where and rhythmic motions of the gut counter dry mouth products such most scleroderma patients have muscles (also known as peristal- as Biotene® mouthwash and oral problems, and fortunately, it has sis). The primary events that cause gel (more information available at been the area where the most trouble in the scleroderma gut are www.biotene.com). Other available advances have been made in ther­ due to a decrease in the blood sup- over-the-counter therapies include apy. The most common symptom ply to the , which are needed xylitol-containing products. Seeing is heartburn (sensation of burn­ to stimulate the bowel. There is also a dentist on regular basis is recom­ ing behind the bone, also some evidence that damage mended. Prescription medications known as GERD [gastroesophageal may occur due to an autoimmune such as Evoxac® and Salagen® may reflux disease]), which is due to process. With decreased stimula- be helpful. the irritation of esophagus by tion, there is progressive weakening regurgitating (backing up or reflux) of muscle strength and tone and ESOPHAGUS (FOOD PIPE) from the . Other symptoms resultant slowing and discoordi- The esophagus transports food to may include dry cough at night, nated motion of the gut. Virtually the stomach by coordinated con­ recurrent chest pain behind the every gut symptom is the result of tractions of its muscular lining. breast bone, persistent hoarse voice, weakening of the gut muscle. The This process is usually gradual and asthma (wheezing and shortness weakening starts in the esophagus automatic and people are usually of breath), regurgitation of food (food pipe) and stomach, and works not aware of it, except when they (expulsion of undigested food from its way down to the small and large swallow something too large, try to the mouth), difficulty swallowing, intestine. eat too quickly, or drink very hot or mouth ulcers and acid taste in the This article will explain the very cold liquids. They then feel the mouth. reasons for the gastrointestinal movement of the food or drink down Reflux is caused by weakening of symptoms and discuss treatments the esophagus into the stomach, the gut muscle valve between the as well. which may be an uncomfortable stomach and esophagus, allowing sensation. acid to “splash” back up into the OROPHARYNGEAL (MOUTH) Approximately 20 percent of Figure 1: Gut Involvement people with scleroderma develop secondary Sjogren’s syndrome, a syndrome associated with dry eyes Large Intestine and dry mouth. This may cause dif- ficult swallowing, gum disease or Esophagus cavities. If people have symptoms Small of dry mouth, they should see a Intestine dentist at least every six months (for some, a visit every four months Stomach may be required). In addition, use sugar-free lozenges to keep your

2 esophagus. The acid, while tracking back into the food pipe, can some­ Table 2: Anti-Reflux Measures times go into the . Some clini­ • Keep head of the bed elevated, 6–8 inches (i.e. wedge pillow, blocks cal studies have even suggested that under head of bed, electric bed). the acid going into the lungs may • Take your biggest meal of the day at noon, small meals otherwise. cause inflammation in sclero­ • Avoid or fluids two hours before bedtime. derma. In addition, weakening of the esophageal muscles themselves • Take frequent small meals (5–6 per day). results in less efficient “milking” of • Do NOT use extra pillows. No tight garments around . (Both of them the food down the esophagus, and may increase pressure over your stomach when bending at the waist at times food can even get tempo­ causing reflux symptoms.) rarily “stuck” in the esophagus and • Take anti-secretory and pro-motility agents (Table 2). patients may need to vomit to clear • Stop smoking (if currently smoking). the esophagus. • Avoid or minimize acid producing foods (, chocolate, coffee). Diagnosis REMEMBER: Certain medications such as nifedipine (taken for Raynaud) can weaken esophagus-stomach causing reflux symptoms. As Diagnosis is based on symptoms. always a balance of treatment should be achieved. Most physicians will first treat these symptoms with medications that can help decrease the acid of the food pipe due to chronic acid improve reflux symptoms and also production. If the symptoms con- damage). minimize the risk of aspiration. tinue, tests such as barium swallow, Why do we need to treat reflux? Medications and esophageal manom- The first and foremost reason to etry can be performed. The barium inhibitors (PPIs) treat reflux is to relieve symptoms The PPIs are medications that sup- swallow requires the person to swal- and improve your quality of life. press acid production. The goal of low a liquid, which contains barium As mentioned before, acid reflux the treatment is to completely stop mixed in a drink, and then take an can also lead to lung inflammation the symptoms of heartburn because X-ray of their esophagus and stom- and even scarring of the lung tissue. continuing symptoms, even if occa- ach. Because these organs are nor- Continuous high acid production sional, reflect ongoing acid produc- mally not visible on X-rays, barium for a long period of time, when tion. To achieve this, higher than temporarily coats the lining of the the esophagus-stomach sphincter recommended doses of PPIs are esophagus, stomach and intestine, is weak, can cause inflammation sometimes used (Table 3). Another making the outline of these organs and scarring of the esophagus piece of advice for people with visible on the X-ray pictures. This (also known as stricture), which scleroderma and reflux is to NEVER process helps to look for the motility leave home without the PPIs, as can lead to Barrett esophagus (a or gut muscle integrity and ulcers missing a couple of doses can cause precancerous condition). (breakdown in the lining of the an increase in acid production and Periodic endoscopic examinations gut) in the esophagus and stomach. rebound of severe symptoms. PPIs to look for early warning signs of Esophageal manometry requires a should be preferably taken 30 min­ cancer are generally recommended small tube be put through the nose utes before breakfast and dinner for people who have Barrett into the esophagus. The tube (if needed). esophagus, an approach referred is built to sense pressure, so it can Recent studies have indicated to as surveillance. an increased risk of diarrhea (may measure sequential muscle con- rarely cause Clostridium difficile tractures (the “milking” motion), Therapy infection), , dementia, and evaluate the motility and func- The treatment of acid reflux and chronic diseases with tion of the esophageal muscles and requires both lifestyle changes and long term use of PPIs. Therefore, the esophagus-stomach sphincter. medications that can you should be aware of these and Endoscopy requires a gastroenter- 1. Decrease acid production. discuss with your physician. The ologist to put a small camera at 2. Increase the motility of the gut benefits still outweigh risks in many the end of a small tube (the endo- muscle. people with scleroderma. Some scope) through your mouth into the people try certain over the coun­ Lifestyle changes esophagus and stomach to look for ter medications, such as Zantac®, damage caused by the acid, such Table 2 goes over the lifestyle Pepcid® (known as H2 blockers) as ulcers and strictures (narrowing changes that may help significantly which may provide temporary relief,

3 the reflux symptoms otherwise the blood vessels can cause anemia Table 3: Medications damage to the esophagus will con- (low count). The per­ Some of the medications used to treat gut tinue and the stricture will return. son may or may not have stomach problems in scleroderma: The stricture, in fact, may require symptoms and may only feel VERY ACID-SUPPRESSING AGENTS Proton-pump inhibitors repeated dilations to keep it open, tired and fatigued. • Prilosec® (omeprazole) 20-40 mg 1 to 2 times per day but the need to redilate usually • Prevacid® (lansoprazole) 15-30 mg 1 to 2 times per day • Aciphex® () 20 mg 1 to 2 times per day decreases over time. Surgery due to Diagnosis • Protonix® (pantorazole) 40 mg 1 to 2 times per day “non-functioning” esophagus should can be diagnosed • Nexium® () 20-40 mg 1 to 2 times per day • Dexilant® (dexilansoprazole) 30-60 mg once per day be the last resort as it often does using the barium study, as dis- H-2 blocker (Over the counter) not work very well. This should only cussed in the esophagus section. • Pepcid®, Tagamet®, Zantac®, Axid® I at night and be considered after a lengthy discus­ as needed if on maximum doses of proton-pump inhibitors Also, a gastric emptying study can sion with your rheumatologist. The PRO-MOTILITY AGENTS be performed in which a person • Reglan® (metaclopromide) 10 mg 3 to 4 times per day† procedure should be performed by eats a meal (e.g. a sandwich or • E-Mycin® () 250-333 mg 3 to 4 times per day surgeons who have experience in • ®* 10-20 mg 3 to 4 times per day egg) which contains a very small • Sandostatin® (octreotide)** 50 mcg, 1 to 2 times per day scleroderma. amount of radiolabeled material SUPPRESSING ANTIBIOTICS • Amoxil®, Trimox® (amoxicillin) 500 mg 3 times per day (that is safe) followed by X-rays • Augmentin® (amoxicillin/ clavulanate) 500/125 or 875/125 STOMACH mg 2 times per day Stomach symptoms in scleroderma of their stomach. The doctors are • Cipro® (ciprofloxacin) 500 mg 3 times per day trying to see how long it takes • Flagyl® (metronidazole) 500 mg 3 times per day are due to slow emptying of the food • Doxy-100® (doxycyline) 100 mg 2 times per day (also known as gastroparesis) into the food to empty into the small • Bactrim® Double Strength 1 tablet 2 times per day • (trimethoprim-sulfamethoxazole) the . The retention of bowel. In scleroderma, the food and • Xifaxan® (rifaximin) 400 mg up to 3 times per day radio­labeled material remain in • Neomycin 500 mg 2 to 4 times per day food in the stomach leads to a sen- the stomach longer than normal LAXATIVES@ sation of nausea, , fullness, • Colace® ( sodium) 100 mg 1 to 2 times per day or bloating sensation and disten­ (sometimes hours) before emptying • Lactulose 15-30 ml 1 to 2 times per day • Milk of magnesia 30-60 ml once per day tion. In some people with sclero- completely. In a person with normal • Senna® (sennosides) 1 capsule 1 to 2 times per day stomach function, approximately 50 • Amitizia® () 8-24 ug 2 times per day derma, the stomach can also have † has a black box warning. Rare cases of tardive dyskinesia, a rare telangiectasias (dilated blood ves­ percent of food is remaining in the movement disorder with prolonged use. sels) lining the walls of the stomach. stomach after 90 minutes. Other This is also known as “watermelon radiographic test includes a wireless but are usually not very effective in motility capsule which is a single- controlling the reflux symptoms and stomach” due to its appearance on take PPIs as “on-demand” therapy. endoscopy. Slow and intermittent use, orally ingested, non-digestible, Some physicians use combinations or rapid bleeding from these dilated capsule with data recorder. This can of the PPIs and H2 blockers. The prokinetic medications (those Figure 2: Watermelon Stomach that improve motility of the gut muscles) can also be used if the symptoms of reflux continue even after reaching maximum doses of PPIs (Table 3). The prokinetic medi- cation should be taken one hour before the meal so the medication is “on board” when one eats. Surgery As mentioned before, the treat- ment of the stricture (which is the scarring of the esophagus due to repeated acid damage) may require dilatation of the narrowing of esophagus during an endoscopy procedure. Progressively longer tubes are gently passed through the narrowed areas to stretch it and open it up. This helps improve the downward motion of the food. This MUST be followed by good control of

4 assess the gastric emptying time but also help with monitoring small and large bowel motility as dis­ Three common causes of cussed below. weight loss or inability to gain Diagnosis of “watermelon stomach,” with its characteristic weight are: gastroparesis, small appearance, can be made by an experienced gastroenterologist using bowel bacterial overgrowth and endoscopy (Figure 2). untreated depression. Treatment The treatment of gastroparesis symptoms of diarrhea with foul people may require longer courses. requires similar measures as smelling stools, which may be oily Any antibiotic may be tried based mentioned in Table 2, and use of or hard to flush. on previous response, side effects prokinetic medications mentioned Another small bowel symptom and coverage by the insurance. in Table 3. For “watermelon stom- that occurs in scleroderma is pseudo Rifaximin and neomycin are oral ach,” use of an argon laser to coagu­ (false)-obstruction. In this condition, antibiotics that have minimal sys­ late or burn the bleeding vessels the bowel is not physically blocked temic absorption. Usually a person can lead to improvement in the ane­ as in true bowel obstruction but has notices improvement in the symp­ mia and symptoms. Patients may just “had enough” and stops work- toms after a course of treatment require multiple laser treatments ing. This is caused by weakening and may notice a slow weight gain. (every two to four weeks) before the of the gut muscle. The patient com- Very often, the treatment has to be bleeding stops completely. plains of belly pain, distention, vom- repeated every month or so to keep iting, and inability to “pass gas.” the bacteria level low. Occasionally SMALL INTESTINE (SMALL treatment must be continuous BOWEL) Diagnosis and some physicians use different The small bowel is the place Bacterial overgrowth is usually antibiotics every month to prevent where we gain most of the diagnosed based on symptoms and the bacteria from becoming resis­ from our food. The food is pushed treated accordingly. If the symp- tant. Pro motility agents (Table 3) from the stomach into the small toms persist after treatment, a can also help. Patients may also bowel by the rhythmic motions of breath test can be performed. In obtain symptomatic relief in the the gut. Once in the small bowel, this, a person ingests sugar (lac­ bacterial overgrowth syndrome by the nutrients and are tose), which is broken down by the regular use of yogurt containing extracted from the food and the bacteria in the small bowel and the bowel-friendly bacteria. Probiotics waste is propelled into the large by-product, hydrogen, is detected are bacteria that have a beneficial bowel and excreted as stool. The in the breath. The physician should effect in the prevention and treat- symptoms of bowel involvement are also evaluate deficiencies ment of gut problems when they are due to lack of muscle tone, leading such as , D and folic ingested. Florastor™, Culturelle™, to stagnation of food in the small acid. and Align™ are three standardized and large bowel. Diagnosis of pseudo-obstruction choices (one or two doses daily) that In the small bowel, the symptoms requires a plain X-ray and a CT can be used. Care should be taken are similar to gastroparesis (bloat­ scan of the . The CT scan not to use probiotics if you are tak- ing sensation and distention, nau­ detects whether the symptoms are ing immunosuppressive therapies sea and vomiting), but abdominal due to a tired “gut,” one that needs as probiotics can rarely cause blood pain may also occur. In addition, rest, or there is something obstruct- infection from live bacteria. Also, due to stagnation of food, the bacte­ ing the small bowel. there is lack of high quality pub­ ria, which normally reside in small lished data to support their efficacy. quantities in the small bowel, can Treatment Sandostatin® (octereotide) has multiply in the food causing “bacte- Bacterial overgrowth is treated been successfully used during rial overgrowth syndrome.” These using antibiotics to reduce the attacks of pseudo-obstruction, and bacteria compete with nutrients overgrowth of bacteria in the small if attacks are frequent, can some- and vitamins causing vitamin defi- bowel. This is achieved by using times be used on a regular basis to ciencies, weight loss, and inability antibiotics for 10 to 14 days at prevent any further attacks. Once to gain weight. There may also be a time (Table 3), although some treated, a person should take

5 prescribed multivitamins to prevent the same as the common diverticu­ body. About 10 percent of sclero- or treat vitamin deficiencies. losis that you often hear about). derma patients may have liver Pseudo-obstruction usually Diverticulitis usually presents with involvement, known as primary requires hospitalization and com­ belly pain and fever. People can also biliary . If this uncommon plete rest of the small bowel. The get diarrhea due to impacted stool. complication occurs, it usually person is given nutrients through occurs 10 to 15 years after the onset Diagnosis the along with pro-motility of scleroderma. Some patients may Diagnosis usually starts with symp- agents. develop biliary cirrhosis before a toms related to the colon. A barium Venting is also performed when diagnosis of scleroderma. Symptoms study can help look at the large needed. “Venting” means to put a usually consist of itchy skin and bowel. Other radiographic tests small surgical tube into the jeju- fatigue. The diagnosis can be made include radiopaque markers and num (small intestine) to aspirate by blood tests for anti-mitochondrial motility capsule. out the air. This keeps the patient and anti- antibody from becoming severely distended Treatment and the treatment consists of doses while in treatment. Total paren- Treatment for constipation requires of ursodeoxycholic acid. teral nutrition (TPN) [high energy liberal use of water and laxatives liquid diet through the veins] may that stimulate the nerve endings in DIET AND SCLERODERMA be prescribed by your physician to the gut wall, which make the mus- Eating Well with Scleroderma give your bowel a prolonged rest cles in the intestine contract with (www.scleroderma.org/eatingwell) and help provide you with essen- provides important information tial nutrition and energy. In many more force. A high fiber diet without regarding diet that may help or patients, the bowel slowly recovers a laxative is not helpful as it just worsen gastrointestinal symptoms. after two to three months, and peo­ adds to the amount of waste the bowel is struggling with. The person ple can go back to eating HOW TO MEASURE GUT regular food. can take a laxative every other day to maintain a healthy bowel regi- INVOLVEMENT AND SEVERITY IN DAY-TO-DAY CARE LARGE INTESTINE (COLON) men. Other laxatives are included Researchers have developed The main function of the large in Table 3. a 34-item questionnaire that bowel is to reabsorb water and salts can assess gut involvement that have been secreted by the rest and severity. The question­ of the gut. This helps the forma- Stool incontinence (symptom of naire is called the University of tion of stools and helps to move the accidentally soiling the underwear California-Scleroderma Clinical waste along. In scleroderma, there before being able to get to a bath- Trial Consortium Gastrointestinal is, as is the case with the rest of the room) occurs in up to a third of peo- Instrument. It has seven scales: five gut, weakening of the gut muscles ple with scleroderma. This is due to scales assess symptoms - reflux, and impaired motility. This can lead the weakening of the rectal muscle distention/bloating, diarrhea, fecal to constipation. Constipation means and poor control over the rectal different things to different people. sphincter. can soilage (or rectal incontinence), and For many people, it simply means assess function of this sphincter. constipation, and two scales assess infrequent stools. Medically speak­ Biofeedback therapy may be helpful the impact of gut symptoms on the ing, constipation usually is defined by improving voluntary squeezing emotional well-being and social as fewer than three bowel move­ of the rectal muscle. Major sclero- functioning. All scales are scored ments per week. Severe constipation derma centers offer classes to teach from 0 (no symptoms or impact on is defined as less than one bowel people this technique. Surgery by quality of life) to 3 (severe symp­ movement per week. This is usually an experienced surgeon might also toms or poor quality of life) except associated with pain and a feeling of be helpful. Preliminary studies diarrhea and constipation scales incomplete emptying of the bowel. have shown promise of sacral nerve that ranges from 0-2 and 0- 2.5, Also, one may get wide-mouthed stimulation (nerves that control rec- respectively. The questionnaire diverticula (large pouches) in the tal tone using a device [Interstim®) takes approximately 5-7 minutes to colon. These do not ordinarily cause in decreasing episodes of rectal complete and is available free online any symptoms. However, on rare incontinence. at http://bit.ly/sctcgit. occasions, the stool can get impact­ The log-in takes less than a min­ ed in them causing inflammation of LIVER ute and asks optional questions the diverticula, called diverticulitis The liver plays an important role regarding your name and e-mail (this is similar to, but not exactly in the detoxification of drugs in our address. These questions are asked

6 so you may receive future e-mails on new developments regarding this Table 4: GI Questionnaire questionnaire and invitations for • High Reflux scale score➔ anti-reflux, PPIs, and promotility (if needed) future studies. In addition, the log- ➔ in screen asks for your age, gender, • High Distention/Bloating scale score Consider delayed gastric emptying and if you have scleroderma. The or bacterial overgrowth ➔ questionnaire is available in 34 lan­ – PLUS Diarrhea trial of antibiotics – No Diarrhea➔ trial of promotility agents guages. The questionnaire can be used by a person to assess their GI • High Constipation scale score➔ stimulant laxatives, good bowel regimen health and monitor their response • Fecal soilage➔ appointment with a colorectal surgeon to treatment. Each scale is pre­ sented as a green bar (none-to-mild has severe reflux symptoms, mod­ promotility agents. The person can symptoms), a yellow bar (moderate erate diarrhea, mild distention/ assess improvement or change in symptoms), or red bar (severe-to- bloating, with no fecal soilage and his/ her symptoms by completing very severe symptoms). constipation. the questionnaire at regular inter­ Table 4 provides an example These GI symptoms are lead­ vals. A red bar should also alert the of an approach in using the scale ing to severe emotional and social person to notify their physician of scores for treatment purposes. As distress. Treatment should include worsening symptoms. an example, the patient in Figure 3 anti-reflux measures, PPIs and

Figure 3: GI Questionnaire The UCLA SCTC GIT 2.0 Questionnaire

For an interactive version of the questionnaire please visit http://bit.ly/sctcgit

7 The Scleroderma Foundation’s mission is three-fold: a To help patients and their families cope with scleroderma through mutual support programs, peer counseling, physician referrals, and educational information. a To promote public awareness and education through patient and health professional semi­ nars, literature, and publicity campaigns. a To stimulate and support research to improve treatment and ultimately find the cause and cure of scleroderma and related diseases.

300 Rosewood Drive, Suite 105 Danvers, MA 01923 Phone: 978-463-5843 Fax: 978-777-1313 Info Line: 800-722-HOPE (4673) Email: [email protected] Website: www.scleroderma.org www.facebook.com/sclerodermaUS www.twitter.com/scleroderma www.youtube.com/sclerodermaUS

Text and logo © 2019, The Scleroderma Foundation, Inc. 8 January 2019 – Rev. 4